TERATOMA

Introduction• Teratomas are tumors comprising more than a

single cell type derived from more than one germ layer

• Teratomas range are :• benign• well-differentiated cystic lesions (mature) • solid and malignant (immature)

Pathophysiology• Teratomas are made up of a variety of

parenchymal cell types representative of more than a single germ layer

• Teratoma arise from totipotential cells, which typically are midline or paraxial

• The most common location is sacrococcygeal (57%).

• The frequency of sacrococcygeal teratomas is 1 per 20,000-40,000 births.

Sacrococcygeal Teratoma • Sacrococcygeal teratomas more common in

females than in males with ratio of approximately 3-4:1

• Can be diagnosed in the prenatal period• Complications may occur in utero or during or

after birth• Approximately 10% of sacrococcygeal

teratomas are associated with other congenital anomalies, primarily defects of the hindgut and cloacal region.

Clinical Findings• Sacrococcygeal teratomas not diagnosed

antenatally present in 2 patterns– neonates, who present with a large, predominantly

benign tumor protruding from the sacral area– the newborn may exhibit only asymmetry of the

buttocks or present when aged 1 month to 4 years with a presacral tumor that may extend into the pelvis.

– The latter group is at higher risk for malignancy.

Differential diagnosisSacrococcygeal teratomas should be

differentiated from – neural tube defects (meningoceles or

meningomyeloceles)– epidermoid cysts– anal duct or pilonidal cysts– rectal abscesses– Lymphangiomas– imperforate anus– rectal prolapse.

Workup• Level of serum AFP and beta HCG may

indicating benign or malignant teratomas• CT scanning of the abdomen and pelvis before

surgical exploration can further delineate sacrococcygeal tumor from normal anatomic features.

• Fine-needle aspiration or core biopsy can be used to differentiate benign from malignant mediastinal masses in 90% of cases.

Staging• Type I– tumors are predominantly external, attached to the coccyx,

and may have a small presacral component (45.8%)– No metastases were associated with this group.

• Type II tumors – have an external mass and significant presacral pelvic

extension (34%)– have a 6% metastases rate.

• Type III– tumors are visible externally, but the predominant mass is

pelvic and intraabdominal (8.6%).– 20% rate of metastases was found in this group.

• Type IV – lesions are not visible externally but are entirely presacral

(9.6%) and have an 8% metastases rate.

Treatment• The treatment of mature teratomas is largely

surgical• may benefit from in utero surgery• should be resected electively in the first week of

life, • Complete excision with chevron-shaped buttock

incision, with preservation of the muscles of the rectal sphincter.

• The coccyx always should be resected with the tumor, to prevent recurrency

• Hemorrhage from the middle sacral vessels and hypogastric arteries is the most common complication.